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Was Cavum Septum Pellucidum the cause of intractable seizure in this 17 years old boy with Wilson's disease?
World Neurosurg. 2017 Jun 12;:
Authors: Rahman A, Ul Haque S, Bahadur Bhandari P, Alam S
Abstract
BACKGROUND: Cavum Septum Pellucidum (CSP), which are often found incidentally in a small number of populations, rarely, become symptomatic if enlarged significantly. Wilson's disease (WD) is an uncommon, autosomal recessive, inborn defect in copper metabolism characterized by abnormal accumulation of copper in various tissues, particularly in the liver and the brain. Seizure disorder, though rare both in CSP and WD, may happen in a very small percentage of patients in either of the two.
CASE DESCRIPTION: We report on a case of 17 years old boy, a known patient of Wilson's disease, who developed intractable seizure for a year, which was not controlled with large amount of anti epileptics. Magnetic Resonance Imaging (MRI) revealed enlargement of his pre-existing CSP, which was small and asymptomatic at the time of diagnosis of WD. His WD was in a state of remission while he developed the seizure disorder. Upon endoscopic cyst fenestration, he was relieved of the seizure.
CONCLUSIONS: Symptomatic Cavum Septum Pellucidum is a rare disorder, but the coexistence of Wilson's disease with it is an even rarer incidence. Endoscopic cyst fenestration is a novel procedure that can be highly successful in properly selected cases. To the best of our knowledge, CSP associated with WD has not been reported in any English literature so far. We are presenting this case for its rarity along with the relevant literature review.
PMID: 28619500 [PubMed - as supplied by publisher]
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